Difference between revisions of "Sjögren syndrome"
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Features ([[salivary gland]]):<ref name=pmid19323360/> | Features ([[salivary gland]]):<ref name=pmid19323360/> | ||
*Lymphocytic infiltration - '''key feature'''. | *Lymphocytic infiltration - '''key feature'''. | ||
**"Benign lymphoepithelial lesion"<ref name=pmid15956090> - intraepithelial lymphocytes. | **"Benign lymphoepithelial lesion"<ref name=pmid15956090/> - intraepithelial lymphocytes. | ||
*Viable [[salivary gland]] or [[lacrimal gland]] acini. | *Viable [[salivary gland]] or [[lacrimal gland]] acini. | ||
*+/-[[Plasma cell]]s. | *+/-[[Plasma cell]]s. | ||
Revision as of 18:15, 10 April 2013
Sjögren syndrome, also Sjögren disease, is a disease that keeps rheumatologists busy. Sjögren is also spelled Sjoegren and Sjogren.
The syndrome may be part of another connective tissue disorder, e.g. rheumatoid arthritis, in which case it is called secondary Sjögren syndrome.[1]
General
Clinical - classically:
- Women in 50s.
- Dry mouth (xerostomia).
- Dry eyes (xerophthalmia).
Blood work:[2]
- ANA +ve.
- Anti-SSA (Ro) +ve.
- Anti-SSB (La) +ve.
Microscopic
Features (salivary gland):[2]
- Lymphocytic infiltration - key feature.
- "Benign lymphoepithelial lesion"[3] - intraepithelial lymphocytes.
- Viable salivary gland or lacrimal gland acini.
- +/-Plasma cells.
- +/-Fibrosis.
DDx:
Note:
- Diagnosis is based on clinicopathologic correlation; the histology alone is insufficient.
- Perivascular lymphocytes not important.
Images:
Grading
Lesions can be graded with the Chisholm-Mason classification.[3] It is based on assessing 4 mm2 area of salivary gland tissue and depends on the abundance and aggregation of lymphocytes as follows:[5]
| Grade | Lymphocytes |
|---|---|
| 0 | absent |
| 1 | slight infiltrate |
| 2 | moderate infiltrate or less than one focus † |
| 3 | one focus † |
| 4 | more than one focus † |
† Focus = aggregrate of 50 lymphocytes.
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LOWER LIP, BIOPSY: - SQUAMOUS MUCOSA WITH PARAKERATOSIS. - SALIVARY GLAND WITH A MINIMAL LYMPHOCYTIC INFILTRATE, SEE COMMENT. COMMENT: Clinical and serologic correlation is required. The inflammation corresponds to Chisholm-Mason classification grade 3.
See also
References
- ↑ Celenligil, H.; Kansu, E.; Ruacan, S.; Eratalay, K.; Irkeç, M. (1990). "Characterization of peripheral blood and salivary gland lymphocytes in secondary Sjögren's syndrome.". Ann Dent 49 (2): 18-22. PMID 1703737.
- ↑ 2.0 2.1 "Information from your family doctor. Sjögren syndrome.". Am Fam Physician 79 (6): 472. Mar 2009. PMID 19323360.
- ↑ 3.0 3.1 Ramos-Casals, M.; Font, J. (Nov 2005). "Primary Sjögren's syndrome: current and emergent aetiopathogenic concepts.". Rheumatology (Oxford) 44 (11): 1354-67. doi:10.1093/rheumatology/keh714. PMID 15956090. http://rheumatology.oxfordjournals.org/content/44/11/1354.long.
- ↑ URL: http://emedicine.medscape.com/article/332125-workup#aw2aab6b5b6aa. Accessed on: 24 July 2012.
- ↑ Chisholm, DM.; Mason, DK. (Sep 1968). "Labial salivary gland biopsy in Sjögren's disease.". J Clin Pathol 21 (5): 656-60. PMC 473887. PMID 5697370. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC473887/?tool=pubmed.